New York-Presbyterian/Weill Cornell Medical Center New York, NY, United States
Sandeep Sikerwar, MD1, David Wan, MD2 1New York-Presbyterian/Weill Cornell Medical Center, New York, NY; 2New York Presbyterian Weill Cornell Medicine, New York, NY
Introduction: IgG4-related disease (IgG4-RD) may involve the biliary ducts in approximately 13% of cases, an entity known as IgG4-related sclerosing cholangitis (ISC), and often presents a diagnostic challenge. We report the case of a patient who had been treated for autoimmune pancreatitis (AIP) but subsequently developed recurrent cholangitis with imaging demonstrating a central hepatic mass concerning for cholangiocarcinoma (CCA). After numerous equivocal biopsies, a percutaneous liver biopsy was suggestive of ISC, and she improved with further immunosuppression.
Case Description/Methods: A 64-year-old female presented to an outside facility with abdominal pain and MR abdomen demonstrated findings suspicious for IgG4-RD involving the pancreas and kidneys with associated biliary ductal dilatation. A pancreatic biopsy confirmed a diagnosis of type 1 AIP due to IgG4-RD. She was treated with a 5-month course of steroids however continued to have episodes of recurrent cholangitis. Repeat imaging demonstrated the development of a central hepatic mass with delayed enhancement concerning for a Klatskin’s tumor. Biliary brushings and biopsies of the mass were unrevealing. She was admitted again shortly afterwards at our institution for cholangitis. Labs were notable for elevated transaminases (AST/ALT 432/778), normal bilirubin, elevated alkaline phosphatase (400), and elevated serum IgG4 levels (280). MRCP re-demonstrated an ill-defined central hepatic mass with diffuse biliary ductal irregularity and dilatation. Repeat biopsies of the biliary duct and a lymph node via EUS and ERCP were again unrevealing. She then underwent a percutaneous biopsy of the hepatic mass that was suggestive of a partially treated ISC. She was subsequently started on prednisone and mycophenolate with interval improvement of the hepatic mass and intrahepatic ductal dilatation, but with persistent extrahepatic dilatation.
Discussion: The diagnosis of ISC is often challenging for a number of reasons: imaging may mimic other cholangiopathies such as PSC and CCA, serum markers are nonspecific, and ductal biopsies are of low yield. Though not entirely conclusive, the evidence in this case favors a diagnosis of ISC and demonstrates that in patients with a known diagnosis of AIP, suspicion for ISC should remain high even after multiple negative biopsies. Additionally, liver biopsies may be of higher yield than ductal biopsies in ISC when there is intrahepatic involvement and should be strongly considered in cases of diagnostic uncertainty.
Figure: Figure 1. MR images before and after 6 weeks of immunosuppression with prednisone and mycophenolate (A) Initial ill-defined central hepatic mass, (B) Interval improvement of hepatic mass, (C) Initial extrahepatic ductal dilatation and thickening, (D) Persistent extrahepatic ductal dilatation and thickening
Disclosures: Sandeep Sikerwar indicated no relevant financial relationships. David Wan indicated no relevant financial relationships.
Sandeep Sikerwar, MD1, David Wan, MD2. P1843 - An Enigmatic Case of IgG4-Related Sclerosing Cholangitis versus Cholangiocarcinoma, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.
